Repatha (evolocumab) — Highmark

Homozygous Familial Hypercholesterolemia (HoFH)

Preferred products

age ≥ 18 years if Praluent OR age ≥ 10 years if Repatha
prescribed by or in consultation with a cardiologist OR lipid specialist OR endocrinologist
diagnosis of HoFH supported by EITHER genetic confirmation of two mutant alleles at LDLR, ApoB, PCSK9, or LDLRAP1 gene locus OR (untreated LDL-C > 400 mg/dL OR untreated total cholesterol > 500 mg/dL) AND (cutaneous or tendon xanthoma before 10 years of age OR evidence of HeFH in both parents)
therapeutic failure to a maximally tolerated statin OR statin intolerance defined as either (two separate statin trials with rhabdomyolysis or skeletal-related muscle symptoms resolving upon discontinuation) OR (during any statin course, CK increase ≥ 10× ULN OR LFTs ≥ 3× ULN OR hospitalization due to statin-related adverse event)
current LDL-C > 115 mg/dL if age ≤ 17 years OR > 70 mg/dL if age ≥ 18 years OR > 55 mg/dL if age ≥ 18 years with ASCVD or major ASCVD risk factors
if request is for Praluent, therapeutic failure or intolerance to plan-preferred Repatha
member will continue to receive concurrent lipid-lowering therapies for HoFH

age ≥ 18 years if Praluent OR age ≥ 10 years if Repatha
diagnosis of HoFH prior to therapy supported by either genetic confirmation of two mutant alleles at LDLR, ApoB, PCSK9, or LDLRAP1 OR (untreated LDL-C > 400 mg/dL OR untreated total cholesterol > 500 mg/dL) AND (cutaneous or tendon xanthoma before 10 years of age OR evidence of HeFH in both parents)
prior to start of therapy, member met LDL-C criteria: >115 mg/dL if age ≤17, >70 mg/dL if age ≥18, OR >55 mg/dL if age ≥18 with ASCVD or major risk factors despite maximally tolerated statin
member will continue concurrent lipid-lowering therapies for HoFH
member has experienced a reduction in LDL-C from baseline